Highlights the potential for rapidly progressive and fatal cardiovascular complications in young children with Marfan's syndrome, even in the absence of classic ocular or severe musculoskeletal signs.
Fatal aortic events possible rapidly in atypical pediatric Marfan syndrome; leaves open optimal early surveillance strategies.
It is the purpose of this report to describe the clinical and pathologic findings in a 4-year-old child who demonstrated the complete spectrum of cardiac pathology associated with Marfan's syndrome during a nine-month period prior to her death. The rapidly progressive alterations in the cardiovascular system were accompanied by relatively mild musculoskeletal manifestations and none of the characteristic ocular changes. The diagnosis of Marfan's syndrome was initially suspected at the age of 4 years after the discovery of a cardiac murmur indicating the presence of aortic insufficiency. At this time plain roentgenograms of the chest revealed cardiac enlargement with dilatation of the ascending aorta. At 4 years and 8 months, angiocardiography demonstrated aneurysmal dilatation of the sinuses of Valsalva and the first portion of the aorta as well as a dissecting aneurysm involving the anterior surface of the ascending aorta. Death occurred abruptly one month later as a result of
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FUE LUN WONG (1964) studied this question.
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